Modified Juvenile Spondyloarthritis Disease Activity Index in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry

Hemalatha Srinivasalu; Erin Brennan Treemarcki; Dax G Rumsey; Pamela F Weiss; Robert A Colbert; CARRA Spondyloarthritis Workgroup and the CARRA Registry Investigators

doi:10.3899/jrheum.220509

Modified Juvenile Spondyloarthritis Disease Activity Index in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry

J Rheumatol. 2023 Apr;50(4):532-537. doi: 10.3899/jrheum.220509. Epub 2022 Nov 1.

Authors

Hemalatha Srinivasalu¹, Erin Brennan Treemarcki², Dax G Rumsey³, Pamela F Weiss⁴, Robert A Colbert; CARRA Spondyloarthritis Workgroup and the CARRA Registry Investigators

Collaborators

CARRA Spondyloarthritis Workgroup and the CARRA Registry Investigators:
K Abulaban, A Adams, C Aguiar Lapsia, A Akinsete, S Akoghlanian, M Al Manaa, A AlBijadi, E Allenspach, A Almutairi, R Alperin, G Amarilyo, M Amir, M Amoruso, S Angeles-Han, S Ardoin, S Armendariz, M Asad, L Asfaw, I Balboni, S Ballinger, F Barbar-Smiley, L Barillas-Arias, M Basiaga, K Baszis, S Baxter, E Behrens, S Benseler, L Bermudez-Santiago, W Bernal, T Bigley, B Binstadt, C Black, M Blakley, J Bohnsack, A Boneparth, H Bradfield, J Bridges, E Brooks, M Brothers, H Brunner, Mary Buckley, Meredith Buckley, H Bukulmez, D Bullock, L Cannon, S Canny, V Cartwright, E Cassidy, E Chalom, Joyce Chang, M Chang, Johanna Chang, A Chang-Hoftman, V Chauhan, A Chen, P Chiraseveenuprapund, K Ciaglia, D Co, E Cohen, H Conlon, R Connor, A Cooper, J Cooper, C Correll, R Cron, M Curry, A Dalrymple, T Davis, D De Ranieri, J Dean, C DeCoste, F Dedeoglu, M De Guzman, N Delnay, E DeSantis, M Dhalla, A Dhanrajani, D Dissanayake, J Drew, K Driest, Q Du, K Dunnock, D Durkee, J Dvergsten, A Eberhard, K Ede, C Edens, T El Tal, M Elder, Y Elzaki, S Fadrhonc, C Failing, D Fair, B Feldman, I Ferguson, P Ferguson, E Flanagan, T Klausmeier, L Fogel, E Fox, M Fox, L Franklin, R Fuhlbrigge, J Fuller, S Gagne, T Garcia, D Gerstbacher, M Gilbert, A Gilbert, I Goh, D Goldsmith, K Gonzales, S Gorry, N Goswami, B Gottlieb, T Graham, T Griffin, J Olson, M Guevara, T Hahn, O Halyabar, E Hammelev, L Harel, S Savani, J Harris, O Harry, A Hay, K Hayward, L Henderson, C Bacha, M Henrickson, A Hersh, K Hickey, L Hiraki, M Hiskey, P Hobday, C Hoffart, M Holland, M Hollander, S Hong, M Horwitz, J Hsu, A Huber, A Huberts, J Huggins, L Huie, J Hui-Yuen, M Ibarra, A Imlay, L Imundo, C Inman, A Jackson, K James, G Janow, S Jared, L Nicole Johnson, J Jones, D Kafisheh, P Kahn, N Karan, R Kaur, E Kessler, B Kienzle, S Kim, Y Kimura, D Kingsbury, M Kitcharoensakkul, K Klein, M Klein-Gitelman, A Knight, L Kovalick, S Kramer, C Kremer, T LaFlam, B Lang, L Tate, S Lapidus, A Lasky, C Lawler, E Lawson, R Laxer, Pui Lee, Patricia Lee, T Lee, A Lee, E Leisinger, L Lentini, M Lerman, D Levy, S Li, S Lieberman, L Lim, E Limenis, C Lin, N Ling, A Brown, M Lloyd, M Lo, A Long, M Lopez-Peña, D Lovell, N Luca, S Lvovich, A Lytch, M Ma, J MacMahon, J Madison, A White, M Mannion, C Manos, L Mansfield, C Mariani, B Marston, V Gennaro, T Mason, L McAllister, J McColl, D McCurdy, K McDaniels, J McDonald, E Meidan, E Mellins, L Michalowski, M Miller, D Milojevic, R Mitacek, R Modica, S Mohan, T Moore, K Moore, L Moorthy, E Morgan, A Moyer, B Murante, E Muscal, O Mwizerwa, K Nanda, N Nasah, L Nassi, S Nativ, M Natter, K Nearanz, J Neely, L Newhall, P Nigrovic, J Nocton, B Nolan, K Nowicki, R Oakes, E Oberle, E Ogbu, M Oliver, R Olveda, K Onel, M Orlando, P O'Rourke, J Padam, A Paller, K Schmidt, N Pan, J Pandya, S Panupattanapong, P Patel, J Patel, S Patrizi, S Paul, J Perfetto, M Perron, K Phillippi, C Plasterer, L Ponder, R Pooni, S Prahalad, M Quinlan-Waters, C Rabinovich, J Rafko, H Rahimi, A Rakestraw, K Rampone, S Ramsey, L Ray, Ann Reed, Annelle Reed, H Reid, D Reiff, S Richins, M Riebschleger, M Riordan, M Riskalla, L Robinson, A Robinson, M Rodriquez, D Rogers, T Ronis, M Rosenkranz, N Rosenwasser, B Rosolowski, H Rothermel, D Rothman, E Rothschild, E Roth-Wojcicki, K Rouster-Stevens, T Rubinstein, J Harrison, J Rupp, W Ambler, J Fennell, N Ruth, S Sabbagh, R Sadun, C Sandborg, L Santiago, V Saper, A Sarkissian, L Scalzi, J Schahn, L Schanberg, K Schikler, A Schlefman, W Stewart, H Schmeling, E Schmitt, R Schneider, G Schulert, K Schultz, C Schutt, C Seper, R Sheets, A Shehab, S Shenoi, J Shirley, A Shuster, N Singer, V Sivaraman, E Sloan, C Smith, J Smith, E Smitherman, J Soep, M Son, D Sosna, L Spiegel, J Spitznagle, H Srinivasalu, H Stapp, K Steigerwald, A Stephens, Y Sterba Rakovchik, S Stern, B Stevens, R Stevenson, K Stewart, C Stingl, M Stoll, E Stringer, R Rivas-Chacon, S Sule, J Sullivan, R Sundel, M Sutter, R Syed, G Syverson, A Szymanski, S Taber, A Tambralli, A Taneja, T Tanner, S Tarvin, A Taxter, J Taylor, M Tesher, A Thatayatikom, B Thomas, N Thomas, T Ting, D Toib, K Torok, H To

Affiliations

¹ H. Srinivasalu, MD, Division of Rheumatology, Children's National Hospital, George Washington University School of Medicine, Washington, District of Columbia, and Pediatric Translational Research Branch, National Institute of Arthritis, Musculoskeletal and Skin Diseases, National Institutes of Health, Bethesda, Maryland, USA; hsriniva@childrensnational.org.
² E.B. Treemarcki, DO, Division of Rheumatology, Department of Pediatrics, University of Utah School of Medicine, Salt Lake City, Utah, USA.
³ D.G. Rumsey, MD, MSc, Department of Pediatrics, University of Alberta, Edmonton, Alberta, Canada.
⁴ P.F. Weiss, MD, MSCE, Division of Rheumatology, Children's Hospital of Philadelphia, and Departments of Pediatrics and Epidemiology, University of Pennsylvania Perelman School of Medicine, Philadelphia, Pennsylvania, USA.

Abstract

Objective: To validate the Juvenile Spondyloarthritis Disease Activity Index (JSpADA), and modified versions thereof, in a North American cohort of patients with enthesitis-related arthritis (ERA).

Methods: We utilized the Childhood Arthritis and Rheumatology Research Alliance Registry database ERA cohort to validate the JSpADA and its modifications (JSpADA6-no Schober, no C-reactive protein [CRP]/erythrocyte sedimentation rate [ESR]; JSpADA7-no Schober; and JSpADA7-no CRP/ESR) using the Outcome Measures in Rheumatology principles of face validity, discriminative validity, and responsiveness to change.

Results: There were 51 subjects (64 visits) with complete JSpADA data with a mean age of 13.7 years and disease duration of 30.9 months. Subjects were predominantly White (84.3%), and 56.9% were male and 50% were HLA-B27 positive. The JSpADA showed high correlation with the clinical 10-joint Juvenile Arthritis Disease Activity Score (cJADAS10; r = 0.81), moderate-to-high correlation with physician global assessment (PGA; r = 0.69), and low-to-fair correlation with Childhood Health Assessment Questionnaire (CHAQ; r = 0.22). The modifications of the JSpADA (JSpADA7-no Schober; JSpADA7-no CRP/ESR; and JSpADA6-no Schober, no CRP/ESR) performed similarly with high correlation with cJADAS10 (r = 0.81, 0.79, and 0.80, respectively), moderate-to-high correlation with PGA (r = 0.65, 0.67, 0.64, respectively), and low-to-fair correlation with CHAQ (r = 0.35, 0.34, 0.39, respectively). All modified versions of JSpADA had good responsiveness to change. All versions of JSpADA had excellent discriminative validity.

Conclusion: We propose the term modified JSpADA for the modification of JSpADA with 6 elements (JSpADA6-no Schober, no CRP/ESR). This shorter disease activity index may improve implementation of JSpADA in both clinical practice and research trials.

Keywords: disease activity score; juvenile idiopathic arthritis; spondyloarthropathy.

Publication types

Research Support, N.I.H., Intramural

MeSH terms

Adolescent
Arthritis, Juvenile* / diagnosis
C-Reactive Protein
Child
Female
Humans
Male
Registries
Rheumatology*
Severity of Illness Index
Spondylarthritis* / diagnosis

Substances

C-Reactive Protein

Grants and funding

Z99 AR999999/ImNIH/Intramural NIH HHS/United States